Definition

What is primary progressive aphasia?

Primary Progressive Aphasia (PPA) is a neurological syndrome in which language capabilities become slowly and progressively impaired. Unlike other forms of aphasia that result from stroke or brain injury, PPA is caused by neurodegenerative diseases, such as Alzheimer’s Disease or Frontotemporal Lobar Degeneration. PPA results from deterioration of brain tissue important for speech and language. Although the first symptoms are problems with speech and language, other problems associated with the underlying disease, such as memory loss, often occur later.

PPA commonly begins as a subtle disorder of language, progressing to a nearly total inability to speak, in its most severe stage. The type or pattern of the language deficit may differ from patient to patient. The initial language disturbance may be fluent aphasia (i.e., the person may have normal or even increased rate of word production) or non-fluent aphasia (speech becomes effortful and the person produces fewer words). A less common variety begins with impaired word-finding and progressive deterioration of naming and comprehension, with relatively preserved articulation.

As with aphasia that results from stroke or brain trauma, the manifestations of PPA depend on what parts of the left hemisphere are relatively more damaged at any given point in the illness. The person may or may not have difficulty understanding speech. Eventually, almost all patients become mute and unable to understand spoken or written language, even if their behavior seems otherwise normal.

How common is primary progressive aphasia?

Please discuss with your doctor for further information.

Symptoms

What are the symptoms of primary progressive aphasia?

Signs and symptoms of primary progressive aphasia (PPA) usually begin gradually and vary based on the type of PPA present. In addition to speech problems, reading and writing also are usually affected. Symptoms generally worsen over time. Affected people may become mute and eventually lose the ability to understand written or spoken language. Although additional cognitive symptoms may emerge later in the course of the illness, deficits must be generally confined to the area of language for at least a couple of years to fulfill a diagnosis of PPA. People with the semantic dementia type may have difficulty with:

Understanding spoken or written language, particularly single words

Understanding word meanings

Naming objects

People with the lopogenic type may have:

Difficulty retrieving words in speech

Frequent pauses in speech while searching for words

Slow speech

Difficulty repeating phrases or sentences

People with the progressive non-fluent type may have:

Difficulty speaking

Hesitant, halting speech

Errors in speech sounds

Difficulty understanding sentences

Difficulty using grammar correctly

Some people with PPA are ultimately diagnosed with other neurodegenerative diseases.

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes primary progressive aphasia?

Primary progressive aphasia is caused by a shrinking (atrophy) of the frontal, temporal or parietal lobes in the brain, primarily on the left side. The condition affects the language centers in your brain.

Scar tissue and abnormal proteins also might be present, and brain activity might be reduced.

Risk factors

What increases my risk for primary progressive aphasia?

Having certain gene mutations. Rare gene mutations have been linked to the disorder. If several other members of your family have had primary progressive aphasia, you might be more likely to develop it.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How isprimary progressive aphasia diagnosed?

Worsening communication difficulty without significant changes in thinking and behavior for a year or two is a hallmark of primary progressive aphasia.

Neurological examination

Doctors might conduct a neurological examination as well as a speech-language evaluation and a neuropsychological evaluation. Tests will measure your speech, language comprehension and skills, recognition and naming of objects, recall, and other factors.

Blood tests

Doctors might order blood tests to check for infections, measure medication levels or look for other medical conditions. Genetic tests can determine if you have genetic mutations associated with primary progressive aphasia or other neurological conditions.

Brain scans

MRI scans can help diagnose primary progressive aphasia, detect shrinking of certain areas of the brain and show which area of the brain might be affected. MRI scans can also detect strokes, tumors or other conditions that affect brain function.

Single-photon emission computerized tomography or PET scans can show blood flow or glucose metabolism abnormalities in areas of your brain.

How is primary progressive aphasia treated?

Primary progressive aphasia can’t be cured, and there are no medications to treat it. However, some therapies might help improve or maintain your ability to communicate and manage your condition.

Speech and language therapy

Working with a speech-language pathologist, focusing primarily on efforts to compensate for eroding language skills, can be helpful. Although speech and language therapy hasn’t been proved to slow progression of the condition, it can help you manage your condition.